FBN2 Antibody (PACO62679)
- SKU:
- PACO62679
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IF
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | FBN2 Antibody (PACO62679) |
抗体コード: | PACO62679 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, IF |
推奨される希釈: | ELISA:1:2000-1:10000, IF:1:200-1:500 |
反応性: | Human |
免疫原: | Recombinant Human Fibrillin-2 protein (304-484AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | Immunofluorescence staining of U251 cells with PACO62679 at 1:200, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
バックグラウンド: | Fibrillin-2: Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2-containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-β bioavailability and calibrating TGF-β and BMP levels, respectively. |
シノニム: | Fibrillin-2 [Cleaved into: Fibrillin-2 C-terminal peptide], FBN2 |
UniProt Protein Function: | FBN2: Fibrillins are structural components of 10-12 nm extracellular calcium-binding microfibrils, which occur either in association with elastin or in elastin-free bundles. Fibrillin-2- containing microfibrils regulate the early process of elastic fiber assembly. Regulates osteoblast maturation by controlling TGF-beta bioavailability and calibrating TGF-beta and BMP levels, respectively. Defects in FBN2 are the cause of distal arthrogryposis type 9 (DA9). A form of distal arthrogryposis, a disease characterized by congenital joint contractures that mainly involve two or more distal parts of the limbs, in the absence of a primary neurological or muscle disease. DA9 is a connective tissue disorder characterized by contractures, arachnodactyly, scoliosis, and crumpled ears. Belongs to the fibrillin family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:Secreted; Secreted, signal peptide Chromosomal Location of Human Ortholog: 5q23-q31 Cellular Component: extracellular matrix; proteinaceous extracellular matrix; microfibril; extracellular region Molecular Function:protein binding; extracellular matrix structural constituent; calcium ion binding Biological Process: positive regulation of osteoblast differentiation; extracellular matrix disassembly; extracellular matrix organization and biogenesis; anatomical structure morphogenesis; positive regulation of bone mineralization; embryonic limb morphogenesis Disease: Macular Degeneration, Early-onset; Arthrogryposis, Distal, Type 9 |
NCBI Summary: | The protein encoded by this gene is a component of connective tissue microfibrils and may be involved in elastic fiber assembly. Mutations in this gene cause congenital contractural arachnodactyly. [provided by RefSeq, Jul 2008] |
UniProt Code: | P35556 |
NCBI GenInfo Identifier: | 66346695 |
NCBI Gene ID: | 2201 |
NCBI Accession: | NP_001990.2 |
UniProt Secondary Accession: | P35556,Q59ES6, B4DU01, |
UniProt Related Accession: | P35556 |
Molecular Weight: | 157,690 Da |
NCBI Full Name: | fibrillin-2 |
NCBI Synonym Full Names: | fibrillin 2 |
NCBI Official Symbol: | FBN2 |
NCBI Official Synonym Symbols: | CCA; DA9 |
NCBI Protein Information: | fibrillin-2; fibrillin 5 |
UniProt Protein Name: | Fibrillin-2 |
Protein Family: | Fibrillin |
UniProt Gene Name: | FBN2 |
UniProt Entry Name: | FBN2_HUMAN |
Antibodies | ELISA Kits |
Anti-FBN2 Antibody (CAB9414) | Human FBN2 / Fibrillin 2 ELISA Kit |
Human Fibrillin-2 (FBN2) ELISA Kit |
Secondary Antibody |
Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014) |
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